{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T21:01:35Z","timestamp":1792789295383,"version":"4.5.0"},"reference-count":65,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2025,9,15]],"date-time":"2025-09-15T00:00:00Z","timestamp":1757894400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"European Union\u2019s Horizon 2020 Framework Programme for Research and Innovation \u2018Brain Involvement in Dystrophinopathies\u2019 (BIND)","award":["847826"],"award-info":[{"award-number":["847826"]}]},{"name":"European Union\u2019s Horizon 2020 Framework Programme for Research and Innovation \u2018Brain Involvement in Dystrophinopathies\u2019 (BIND)","award":["#M16SO1\/276330"],"award-info":[{"award-number":["#M16SO1\/276330"]}]},{"name":"ECOS Nord-Conahcyt","award":["847826"],"award-info":[{"award-number":["847826"]}]},{"name":"ECOS Nord-Conahcyt","award":["#M16SO1\/276330"],"award-info":[{"award-number":["#M16SO1\/276330"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Duchenne muscular dystrophy (DMD) is a severe neuromuscular disease caused by mutations in the DMD gene, leading to muscle degeneration and shortened life expectancy. Beyond motor symptoms, DMD patients frequently exhibit brain co-morbidities, linked to loss of brain-expressed dystrophin isoforms: most frequently Dp427 and Dp140, and occasionally Dp71 and Dp40. DMD mouse models, including mdx5cv and mdx52, replicate key aspects of the human cognitive phenotype and recapitulate the main genotypic categories of brain phenotype. However, the spatio-temporal expression of brain dystrophin in mice remains poorly defined, limiting insights into how its deficiency disrupts brain development and function. We systematically mapped RNA and protein expression of brain dystrophin isoforms (Dp427 variants, Dp140, Dp71, and Dp40) across brain regions and developmental stages in wild-type mice. Dp427 isoforms were differentially expressed in the adult brain, with Dp427c enriched in the cortex, Dp427p1\/p2 in the cerebellum, and Dp427m was also detected across specific brain regions. Dp140 was expressed at lower levels than Dp427; Dp71 was the most abundant isoform in adulthood. Dp140 and Dp71 displayed dynamic developmental changes, from E15 to P60, suggesting stage-specific roles. We also analysed mdx5cv mice lacking Dp427 and mdx52 mice lacking both Dp427 and Dp140. Both models had minimal Dp427 transcript levels, likely due to the nonsense-mediated decay, and neither expressed Dp427 protein. As expected, mdx52 mice lacked Dp140, confirming their genotypic relevance to human DMD. Our study provides the first atlas of dystrophin expression in the wild-type mouse brain, aiding understanding of the anatomical basis of behavioural and cognitive comorbidities in DMD.<\/jats:p>","DOI":"10.3390\/cells14181441","type":"journal-article","created":{"date-parts":[[2025,9,15]],"date-time":"2025-09-15T16:27:33Z","timestamp":1757953653000},"page":"1441","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Regional Expression of Dystrophin Gene Transcripts and Proteins in the Mouse Brain"],"prefix":"10.3390","volume":"14","author":[{"given":"Konstantina","family":"Tetorou","sequence":"first","affiliation":[{"name":"The Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"},{"name":"National Institute for Health Research, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-2417-8838","authenticated-orcid":false,"given":"Artadokht","family":"Aghaeipour","sequence":"additional","affiliation":[{"name":"The Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"},{"name":"National Institute for Health Research, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shunyi","family":"Ma","sequence":"additional","affiliation":[{"name":"The Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"},{"name":"National Institute for Health Research, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Talia","family":"Gileadi","sequence":"additional","affiliation":[{"name":"The Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"},{"name":"National Institute for Health Research, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amel","family":"Saoudi","sequence":"additional","affiliation":[{"name":"Centre National de la Recherche Scientifique (CNRS), Institut des Neurosciences Paris-Saclay, Universit\u00e9 Paris-Saclay, 91400 Saclay, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pablo","family":"Perdomo Quinteiro","sequence":"additional","affiliation":[{"name":"Human Genetics Department, Leiden University Medical Center, 2333 Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge","family":"Arag\u00f3n","sequence":"additional","affiliation":[{"name":"Centre National de la Recherche Scientifique (CNRS), Institut des Neurosciences Paris-Saclay, Universit\u00e9 Paris-Saclay, 91400 Saclay, France"},{"name":"Departamento de Gen\u00e9tica y Biolog\u00eda Molecular, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), Mexico City 07360, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0683-8897","authenticated-orcid":false,"given":"Maaike","family":"van Putten","sequence":"additional","affiliation":[{"name":"Human Genetics Department, Leiden University Medical Center, 2333 Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2783-688X","authenticated-orcid":false,"given":"Pietro","family":"Spitali","sequence":"additional","affiliation":[{"name":"Human Genetics Department, Leiden University Medical Center, 2333 Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7186-9016","authenticated-orcid":false,"given":"Cecilia","family":"Montanez","sequence":"additional","affiliation":[{"name":"Departamento de Gen\u00e9tica y Biolog\u00eda Molecular, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), Mexico City 07360, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8783-8185","authenticated-orcid":false,"given":"Cyrille","family":"Vaillend","sequence":"additional","affiliation":[{"name":"Centre National de la Recherche Scientifique (CNRS), Institut des Neurosciences Paris-Saclay, Universit\u00e9 Paris-Saclay, 91400 Saclay, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0375-476X","authenticated-orcid":false,"given":"Jennifer 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UK"},{"name":"National Institute for Health Research, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,9,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1093\/hmg\/1.7.505","article-title":"Expression of Four Alternative Dystrophin Transcripts in Brain Regions Regulated by Different Promoters","volume":"1","author":"Monaco","year":"1992","journal-title":"Hum. 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